Diagnostic criteria for optic neuromyelitis optica spectrum disorders (NMOSD) published in Neurology 2015
AQP4-IgG positivity when
1. at least one core clinical feature.
2. positive for AQP4-IgG (an AQP4 transfected cell-based assay is highly recommended).
3, except for other diagnoses.
AQP4-IgG negative (really not detected) or when no test is available (e.g., grassroots, e.g., patients do not want to be tested for economic reasons)
1. At least two core clinical features (either one at a time or sequential) and all of the following.
a, at least one core clinical feature of optic neuritis, long-segment transverse acute myelitis, or extreme posterior zone syndrome).
b, spatially disseminated (at least two core clinical features).
c, meet the corresponding requirements of MRI (that is, the clinical features should match the corresponding lesions, such as myelitis should have MRI lesions).
2, AQP4-IgG negative or not detectable.
3, except for other diagnoses.
Core clinical features
1, optic neuritis.
2, acute myelitis (these two previous ones are old faces).
3, posterior polar area syndrome: erratic, nausea and vomiting, except for other causes. (Will it be possible to have a reassuring eruption in the future?) ;.
4, acute brainstem syndrome.
5, episodic sleeping sickness or other acute mesencephalic syndrome with a typical mesencephalic lesion of NMOSD.
6, some manifestations of brain damage (feel this sentence is a supporting (fei) role (hua), in fact, the key is to match the lesion), must be consistent with the NMOSD characteristic lesion (if not, it is a small red flag).
MRI requirements (AQP4-IgG negative or a must when there is no condition to measure)
1. Acute optic neuritis: a. Normal brain MRI or only non-specific lesions (I think at least in part to exclude MS, which also has optic neuritis, but brain lesions are relatively common). Or b, T2 or T1 enhancement can be seen with an optic neuropathy longer than 1/2 the full length of the optic nerve or involving the optic cross (this article would be supportive of NMOSD).
2, acute myelitis, ≥ 3 vertebral segments in length (in addition, if the posterior phase, atrophy of this length can be seen, in short, long on one word).
3, acute posterior polar region syndrome: dorsal medulla oblongata/posterior polar region lesion.
4, Acute brainstem syndrome with brainstem lesions near the ventricular canal.
The little red flags
I. Clinical/laboratory indicators
1, Clinical details or laboratory indicators
a, progressive course of the disease (e.g., slowly worsening as in primary progressive multiple sclerosis).
b. Atypical time to peak attack: too fast <4< span=""> hours (likely spinal cord ischemia/infarction), too slow >4 weeks (say, not necessarily nodal disease or tumor).
c. Partial transverse myelitis (spinal cord damage is not brutal enough, e.g. paralysis is limited to unilateral, especially since MRI is not long transverse either!!!) !
d, CSF oligoclonal bands (<20%< span=""> positive for NMO, while MS is >80% positive).
2, with some other diseases that can cause NMOSD-like symptoms
a. Nodal disease: e.g., with mediastinal lymph node enlargement, fever, night sweats, elevated blood ACE or IL-2 receptors.
b, tumors: be alert for lymphomas, paraneoplastic syndromes (especially collapsin response mediator protein-5 related optic neuropathy, myelopathy, anti-Ma related mesencephalic syndrome)
c. Chronic infections, such as HIV and neurosyphilis.
Imaging indicators
1.Brain
a, consistent with MS features (in fact, 16% of NMOSD meet the diagnostic criteria for MS MRI, so it is called a small red flag rather than an absolute exclusion criterion).
① lesion on the vertical lateral ventricular surface.
② lesion in the lower temporal lobe near the anterior horn of the lateral ventricle.
(iii) proximal cortex with involvement of subcortical U-shaped fibers.
(iv) cortical lesions.
b. Looks neither like MS nor NMO.
For example, but not limited to: lesions that persistently (>3 months) strengthen on MRI.
2. spinal cord that looks more like MS
a, T2 sagittal lesions less than three vertebral segment lengths.
b, T2 axial lesions are predominantly (>70%) located in the periphery of the spinal cord (said so around actually the white matter conduction tracts guys).
c. The lesions are diffuse and blurred in T2, like primary progressive multiple sclerosis or multiple episodes of multiple sclerosis fused together.
Acute phase spinal cord MRI
1. long segmental transverse lesions consistent with acute myelitis.
a, T2 sagittal >3 vertebral segments (cliché).
b, predominantly central involvement of the spinal cord (>70% in the gray matter).
c, T1 enhancement scan visible as enhancement.
2, other characteristic lesions.
a, upward invasion of the brainstem if the cervical medulla.
b, swelling of the spinal cord.
c, T2 high signal lesions show low signal on T1.
MRI of the spinal cord in the chronic phase
Long segmental spinal cord atrophy (indicating more complete destruction with clear borders, >3 vertebral segments), T2 may or may not be bright optic nerve MRI unilateral or bilateral optic nerve with signal changes on T2 flat scan or T1 enhancement (long (e.g., >1/2 length), lesion posteriorly, may involve optic cross) brain MRI, T2 visible as.
1. involvement of the dorsal medulla (especially the extreme posterior region), which can be either small and isolated (often bilateral) or contiguous from lesions in the upper cervical segment.
2. brainstem/cerebellum, lesions near the ventricular canal of the four ventricles.
3, lesions near the hypothalamus, thalamus, and triventricular ventricular meninges.
4, unilateral or bilateral, large and fused subcortical or deep white matter lesions.
5, corpus callosum involvement with long (> half the length), diffuse, mixed signal lesions that may be associated with edema.
6. long corticospinal tract lesions, either unilaterally or bilaterally, extending from the internal capsule to the cerebral peduncle.
7. extensive foci near the ventricular canal, which may be accompanied by enhancement.
At least one core clinical feature is optic neuritis, long segmental transverse acute myelitis, or extreme posterior zone syndrome b. Unilateral or bilateral optic nerve with signal changes on T2 scan or T1 enhancement (long (e.g., >1/2 length), lesions posteriorly, may involve the optic cross) involving the dorsal medulla (especially the extreme posterior zone), either small and isolated (often bilateral) or contiguous from lesions in the upper cervical segment.